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Background: Arterial thrombosis (AT) is an increasingly recognized complication in pediatrics. Consensus clinical practice guidelines suggest immediate removal of the indwelling arterial catheter and a short course (5-7 days) of anticoagulation. The optimal duration and modality of antithrombotic therapy in children are yet to be determined., Aims: Describe treatment patterns and outcomes in pediatric patients with AT and explore predictors for complete thrombus resolution or long-term complications., Methods: Single-institution retrospective study. Patients were identified by ICD-9 and ICD-10 codes for the diagnosis of AT or reports of AT on ultrasound from January 1, 2012, to October 1, 2022. Descriptive and logistic regression analyses were used., Results: 101 patients were included. The median age was 2.2 months. The most common underlying diagnoses were congenital heart disease (39.6%) and infection (22.8%). A majority of patients had symptomatic thrombosis in an extremity, and 78% were catheter-associated. 81% of patients received anticoagulation with a median duration of 35 days. Out of the 70 patients who were treated with anticoagulation alone and had a follow-up imaging, 70% had complete resolution after 90 days of anticoagulation. No clear predictors of complete resolution were identified. Eighteen patients had long-term sequelae secondary to arterial insufficiency. Those with infection-associated AT were more likely to have long-term complications. The major and clinically relevant non-major bleeding rate was 11%., Conclusion: Duration of anticoagulation was widely variable, and 70% of patients achieved complete resolution by 90 days of anticoagulation. A significant proportion of patients developed long-term sequelae secondary to arterial insufficiency. Sepsis/infection at the time of diagnosis with AT was more likely to be associated with long-term complications., (© 2023 Wiley Periodicals LLC.)

Background: This study was performed to describe the single-center experience of deep vein thrombosis (DVT) in children with severe traumatic brain injury (sTBI) who were mechanically ventilated with a central line, and to identify potentially modifiable risk factors. It was hypothesized that children with DVT would have a longer duration of central venous line (CVL) and a higher use of hypertonic saline (HTS) compared to those without DVT., Procedure/methods: This was a retrospective study of children (0-18 years) with sTBI, who were intubated, had a CVL, and a minimum intensive care unit (ICU) stay of 3 days. Children were analyzed by the presence or absence of DVT. HTS use was evaluated using milliliter per kilogram (ml/kg) of 3% equivalents. Univariable and multivariable logistic regression models were used to determine which factors were associated with DVT., Results: Seventy-seven children met inclusion criteria, 23 (29.9%) had a DVT detected in an extremity. On univariable analysis, children with DVT identified in an extremity had prolonged CVL use (14 vs. 8.5 days, p = .021) and longer duration of mechanical ventilation (15 vs. 10 days, p = .013). HTS 3% equivalent ml/kg was not different between groups. On multivariable analysis, mechanical ventilation duration was associated with DVT detection in an extremity, whereas neither CVL duration nor HTS use had an association., Conclusions: There was a high incidence of extremity DVT detected in children with sTBI who received invasive mechanical ventilation and had a CVL. HTS administration was not associated with DVT detection in an extremity., (© 2022 Wiley Periodicals LLC.)

Rates of venous thromboembolism have increased in the adolescent population over the last two decades, likely due to advanced diagnostics, increased use of central venous catheters, chronic medical conditions, obesity, and oral contraceptive use. Of these factors, a modifiable risk factor for adolescents is obesity. Sedentary lifestyle and prolonged immobilization are additional prothrombotic risk factors that are often associated with obesity. With ever-increasing screen time, sedentary behavior has risen accordingly, especially among gamers. We present four cases of adolescents who developed life-threatening venous thromboembolic events in the setting of obesity, sedentary lifestyle and/or immobilization, and prolonged video game use., (© 2018 Wiley Periodicals, Inc.)

Objective: While post-thrombotic syndrome (PTS) is increasingly recognized in children with a history of deep vein thrombosis (DVT), its impact on the health-related quality of life (HRQoL) is unknown. Our objective was to evaluate the association between the PTS and HRQoL by surveying a cohort of patients treated at our institution for DVT., Materials/methods: All unique pediatric patients (0-18 years) treated for a DVT at the Mayo Clinic during the 15-year period, 1995-2009 were identified. A previously validated PTS survey instrument and age appropriate Pediatric Quality of Life inventory, version 4 (PedsQL 4.0) were mailed to eligible patients. Linear regression models were fit to compare the HRQoL scores between PTS groups (none, mild, moderate/severe), after adjusting for the presence of potential covariates., Results: Of the 90 respondents, 65 (72%) reported signs and/or symptoms of PTS. Mean age (± SD) at DVT diagnosis and survey completion were 12.8 (± 6.1) and 19.3 (± 7.7) years, respectively. Self-report PedsQL 4.0 module was completed by 79 patients, and 34 guardians completed the parent-proxy module. Patients with moderate to severe PTS reported significantly worse total HRQoL score (mean ± SD, 71.3 ± 13.4) as compared to patients with mild PTS (84.8 ± 14.2) and no PTS (83.4 ± 14) (P = 0.001)., Conclusion: Moderate to severe PTS has a significant impact on self-reported HRQoL as measured using the generic PedsQL 4.0. Further research is warranted to develop a venous disease-specific quality of life measure for children with a history of DVT., (© 2013 Wiley Periodicals, Inc.)

Given the rarity of follicular lymphoma (FL) in children, there is limited data on which to base treatment recommendations. Herein, we report our institutional experience of using rituximab with multiagent chemotherapy for pediatric FL. Six pediatric patients were diagnosed with FL from 2000 to 2009. All patients received rituximab in combination with cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) for varying durations. Five of the six patients remain in remission with a median follow-up of 31 months. Larger randomized trials are indicated to establish the efficacy of this regimen for pediatric FL patients., (Copyright © 2011 Wiley-Liss, Inc.)

Development of pseudotumors is an unusual complication of hemophilia. Treatment is controversial, especially in patients with large proximal lesions. Surgery, while curative, can be associated with massive intra-operative bleeding, infection and amputation. Arterial embolization of blood vessels supplying the pseudotumor may reduce these complications. Herein, we report a 14-year-old patient with moderate hemophilia B with a pelvic pseudotumor and pseudoaneurysm that failed conservative management with factor replacement alone. He was successfully treated with Bead Block and coil embolization followed by surgical extirpation of the lesion 24 hr later., (© 2010 Wiley-Liss, Inc.)

We present the hemostatic management of severe hemophilia A of an infant with high risk of inhibitor development who underwent cardiac surgery for correction of tetralogy of Fallot. Continuous infusion of recombinant factor VIII resulted in good hemostasis during surgery and postoperatively. Unfortunately, the patient had inhibitor development 2 months after cardiothoracic surgery. Although cardiac surgery is successful, exposure to factor concentrates in severe hemophilia early in life might predispose a patient to inhibitor development.

Although typically a disorder of adults, acquired von Willebrand syndrome (AVWS) is increasingly being recognized in the pediatric population in association with congenital cardiac diseases, certain neoplasia, and hypothyroidism. Transplacental transfer of maternal immunoglobulin G (IgG) antibodies as a cause of neonatal disorders in infants born to mothers with autoimmune conditions has been reported. We describe the diagnosis and peripartum clinical management of AVWS due to monoclonal gammopathy of undetermined significance (MGUS) and the first reported case of transient neonatal AVWS due to transplacental transfer of maternal IgG antibodies.

Congenital combined deficiency of the vitamin K-dependent coagulation factors is a rare bleeding disorder caused by either a defect in the gamma-glutamyl carboxylase or the vitamin K epoxide reductase enzyme complex. The diagnosis should be considered when vitamin-K dependent factor activities are decreased and liver dysfunction, vitamin K deficiency, and factitious coumarin ingestion have been excluded. We report a case of VKCFD in a child resulting from compound heterozygosity for two novel splice site mutations of the gamma-glutamyl carboxylase gene. Oral vitamin K supplementation resulted in partial resolution of proteins and complete resolution of bleeding., (Copyright 2009 Wiley-Liss, Inc.)

Catastrophic antiphospholipid syndrome (CAPS) is a life-threatening condition characterized by small-vessel thrombi and a rapid onset of multiorgan system failure associated with systemic inflammatory response syndrome. Current treatment options include anticoagulants, corticosteroids, plasma exchange, and intravenous immunoglobulin, but these are not always effective. Rituximab, a chimeric anti-CD20 monoclonal antibody, may help eliminate autoreactive B cells and thus limit the rapid inflammatory process involved in CAPS. We describe the use of rituximab in the successful initial management of a probable case of pediatric CAPS., (Copyright 2008 Wiley-Liss, Inc.)

Infliximab-daclizumab was used to treat acute and chronic liver and gut graft-versus-host disease (GVHD) in two children after standard immunosuppressive therapy failed. Infliximab (10 mg/kg weekly, 4 doses) and daclizumab (1 mg/kg, days 1, 4, 8, 15, and 22) were given over 1 month. In case 1, grade 2 chronic GVHD of the liver developed 1 year after transplantation and failed to improve with tacrolimus, mycophenolate mofetil, and prednisone. In case 2, corticosteroid-unresponsive grade 3 acute liver and gut GVHD developed on day +37. In both patients, GVHD responded to the infliximab-daclizumab regimen without toxicity and immunosuppressive therapy was discontinued.